Intravenous cyclophosphamide for interstitial lung disease associated to systemic sclerosis: results with an 18-month long protocol including a maintenance phase

Clin Exp Rheumatol. 2007 Mar-Apr;25(2):293-6.


Objective: Cyclophosphamide (CYC) is generally considered the most promising agent available today for systemic sclerosis (SSc)-related interstitial lung disease (ILD). However, the optimal dosage and length of treatment are still undetermined. Our objective was to evaluate the effect of an 18-month long protocol with intravenous (iv) CYC.

Methods: In a single-centre, prospective, observational study, 13 patients with SSc and active alveolitis were given 8 iv pulses in a 6-months period (CYC 750 mg + 6-methylprednisolone 125 mg every three weeks), as an induction therapy. Patients received maintenance therapy with further cycles at 4 (3 pulses), 6 (3 pulses) and 9 weeks (3 pulses) interval. Total CYC dosage was 12.75 g in an 18-month period. End-points were modifications of lung function test (LFT).

Results: During the first 6 months of treatment with CYC an increase in Forced Vital Capacity (FVC; p = 0.005) and in diffusion lung capacity for carbon monoxide (DLCO; p = 0.10) was observed; during the maintenance therapy, there was a stabilization in FVC and a mild, non significant decline in DLCO. Treatment was well tolerated.

Conclusion: iv CYC can induce an initial improvement in LFT (particularly, in FVC) in the first six months, but no further improvement was observed during the maintenance phase.

Publication types

  • Clinical Trial

MeSH terms

  • Adult
  • Antirheumatic Agents / administration & dosage
  • Antirheumatic Agents / therapeutic use*
  • Carbon Monoxide / metabolism
  • Cyclophosphamide / administration & dosage
  • Cyclophosphamide / therapeutic use*
  • Dose-Response Relationship, Drug
  • Female
  • Humans
  • Injections, Intravenous
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / etiology*
  • Lung Diseases, Interstitial / metabolism
  • Male
  • Middle Aged
  • Prospective Studies
  • Pulse Therapy, Drug
  • Respiratory Function Tests
  • Scleroderma, Systemic / complications*
  • Time Factors


  • Antirheumatic Agents
  • Carbon Monoxide
  • Cyclophosphamide